Primary Vaginal Ewing's Sarcoma or Primitive Neuroectodermal Tumor with Liver and Lung Metastasis in a 45-Year Old Woman

CASE REPORT Title - Primary Vaginal Ewing's Sarcoma or Primitive Neuroectodermal tumor with Liver and Lung metastasis in a 45-year old woman. Dr. Prashant Patel, Resident. Dr. Shruti Gohel, Resident. Dr. Ankita Parikh, Associate Professor. Dr. U Suryanarayana, Professor& HOD. Dr. Sonal Patel Shah, Assistant Professor. Dr. Shikha Dhal, Assistant Professor. Dr. Rakesh Vyas, Director. G. C. R. I. , Ahmedabad. - Extra osseous Ewing sarcoma, PNET, Metastasis, Vagina. Introduction - Ewing's Sarcoma/PNET of the female genital tract is very unusual, but has been reported to involve the ovary, uterine corpus, uterine cervix, and vulva. To our knowledge, only 10-12 cases of primary vaginal Ewing's Sarcoma/PNET have been previously reported in the literature and none of them had any evidence of metastasis when reported. Here, we present a rare case of primary vaginal Ewing's Sarcoma/PNET with liver, breast and lung metastasis. Case Report - We present a case of a 45 year old woman, gravida 2, para 2, with who presented with the complaints of whitish, foul smelling vaginal discharge and swelling at vulva since 2 months and itching at the local site since 1 month. Per vaginal& per speculum examination of vagina showed 6X6 cm submucosal growth over left sided vulva, disease extended from 10?O clock to 5?O clock position of middle& lower vagina but cervix was free. Rectal examination revealed b/l paravaginal tissue medially involved but the rectal mucosa was free. Routine haemogram, liver and renal function tests were within normal limits. Chest radiograph revealed no abnormality and Contrast-enhanced Computed Tomography (CECT) of Thorax revealed few calcified nodes in right hilar region and sub-carinal region with bilateral lung metastasis and liver metastasis. CECT Abdomen revealed liver metastasis. CECT Pelvis showed a 57X47X120 mm lesion with internal necrotic area involving vagina more on left side and extending upto labial fold, both ischiorectal fossa and infiltrating the proximal part of the left obturator internus, loss of fat plane with rectum and anal canal, 40X38 mm fibroid involving fundus of uterus, bilateral adnexa were normal with no ascites or lymphadenopathy. Bone scan was normal. Punch biopsy of the vaginal mass was then performed which showed poorly differentiated Adenocarcinoma with probable Neuroendocrine differentiation. Immunohistochemistry was done with a panel of antibodies, which revealed Ewing's Sarcoma. Following our diagnosis of Primary Ewing's sarcoma or PNET of the vagina, our patient was subjected to combination chemotherapy for 35 days 1 cycle VACA (Vincristine, Actinomycin-D, Cyclophosphamide and Doxorubicin). During chemotherapy disease was progressive, so the patient was sent for palliative radiotherapy and was treated with 30Gy/15# (@200cGy/#) by AP/PA portals, during which our patient was found to have clinically progressive disease. Following this, she was on palliative chemotherapy, single agent (Adriamycin). Table- 10 cases of primary vaginal Ewing's sarcoma/PNET reported earlier. Study Age T-size IHC profile Treatment Follow up (months) Outcome Liao et al 30 5 VIM+, MIC2+, FLI+, Synaptopysin+, NSE+, S-100+ TAH+BSO+CT 36 FOD Farley et al 35 4 MIC+ CT+EBRT+ICBT 48 FOD Vang et al 35 3 VIM+, MIC2+ WE+CT+RT 19 FOD Gaona-luviano et al 34 4 MIC2+ WE+CT+RT+ICBT 20 FOD Rekhi ET AL 17 10 VIM=, MIC2=, FL1=, BCL2= CT+EBRT FU Al-Taimini et al 47 ND ND ND ND ND Yip et al 27 6 MIC2+ WE+RT 18 FOD Pang et al 54 4 MIC2+ EBRT+ICBT 18 DOD Petkovic et al 45 9 MIC2+ CT+EBRT+ICBT 18 AWD McCluggage et al 40 8 VIM+, MIC2+, FL1+ ND ND Our case 45 11 VIM+, MIB1+ (greater than50%), CD99+ CT+EBRT+CT FU PNET = primitive neuroectodermal tumour, T-size = tumour size in largest dimension, IHC=immunohistochemistry, VIM = vimentin, + = positive, ? = negative, WE = Wide excision, CT = chemotherapy, EBRT = external beam radiotherapy, ICBT = intracavitary brachytherapy, TAH+BSO = total abdominal hysterectomy + bilateral salpingoophorectomy, MIC2 = Microneme protein 2, FLI1 = FOD = free of disease, AWD = alive with disease, DOD = died of disease, FU = follow-up, ND=not described Result - Our patient was regular in treatment. Discussion- Ewing's sarcoma has a potential for haematogenous metastasis and the most common sites of metastasis include lungs, bones and bone marrow. About 25% of patients have metastatic disease at presentation, patients with isolated lung metastasis have better prognosis than those with extra-pulmonary disease. The chemotherapy regimen and initial treatment for patients with metastatic disease is the same as that for localized disease. At the time of local therapy, all sites of the disease must be re-evaluated. If tumor shows progression or there is persistence of widespread disease, there is little hope for cure and hence, such patients should be treated with palliative intent. For patients responding well, at this stage, local therapy in the form of surgery and/or radiation is recom